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  • Acute rheumatic fever

  • 390 Rheumatic fever without mention of heart involvement
  • 391 Rheumatic fever with heart involvement
  • 392 Rheumatic chorea

  • I00 Rheumatic fever without heart involvement
  • I01.0 Acute rheumatic pericarditis
  • I01.1 Acute rheumatic endocarditis
  • I01.2 Acute rheumatic myocarditis
  • I01.8 Other acute rheumatic heart disease
  • I01.9 Acute rheumatic heart disease, unspecified
  • I02.0 Rheumatic chorea with heart involvement
  • I02.9 Rheumatic chorea without heart involvement


  • Autoimmune inflammatory disease that can develop as a sequela of streptococcal infection, group A
  • Characterized by inflammatory lesions of the joints, heart, blood vessels, subcutaneous tissue, and central nervous system
  • Form of endocarditis

Essentials of Diagnosis

  • Rheumatic fever has variable manifestations; therefore, no specific diagnostic test exists for diagnosis
  • Usually occurs approximately 20 days after strep throat or scarlet fever
  • Diagnosis is based on the Jones criteria; major and minor criteria used to confirm diagnosis
  • Diagnosed if individual meets two major criteria or one major and one minor crieteria with previous strep infection
    • Major criteria
      • Carditis
      • Polyarthritis
      • Sydenham chorea
      • Erythema marginatum
      • Subcutaneous nodules
    • Minor criteria
      • Polyrthralgia
      • Previous rheumatic fever or rheumatic heart disease
      • Fever
      • Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) levels
      • Prolonged PR interval
  • Evidence of preceding group A streptococcal infection: positive throat culture or rapid antigen test result
  • Elevated or rising streptococcal antibody titer

General Considerations

  • The most significant complication is rheumatic heart disease, which usually occurs after repeated bouts of acute illness
    • It can present as valvular stenosis, most commonly involving the mitral valve
    • These patients are prone to infective endocarditis and stroke
  • Chorea can present months after the inciting infection
  • Some physicians monitor ESR and restart activity when it normalizes


  • Rheumatic fever is predominantly a disease of developing countries and is concentrated in areas of deprivation and crowding.
  • The risk of developing rheumatic fever after an episode of streptococcal pharyngitis has been estimated at 0.3 to 3%.
  • Overall incidence in the United States is less than 1 per 100,000.
  • It is most common in 5- to 15-year-old children.
  • A genetic predisposition to rheumatic fever does exist.
  • The disease does not have a major racial or gender predisposition.

Signs and Symptoms

  • Signs and symptoms result from inflammation in the heart, joints, skin, or central nervous system.
  • Sore throat: 35% to 60% of patients with rheumatic fever recall having upper respiratory symptoms in the preceding several weeks.
  • Swollen and painful joints, particularly knees, ankles, elbows, and wrists
  • Erythema marginatum: flat or raised red lattice-like rash ...

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